Akihiro Igata

The Crow‐Fukase syndrome A study of 102 cases in Japan

Clinical manifestations of 102 cases with the Crow-Fukase syndrome (the syndrome of polyneuropathy, anasarca, skin changes, endocrinopathy, dysglobulinemia, and organomegaly), with or without myeloma, were reviewed. Fifty-six cases with myeloma consisted of 31 with osteosclerotic, 17 with mixed osteosclerotic and osteolytic, and 8 with osteolytic. Forty-six cases without myeloma consisted of 2 with extramedullary plasmacytoma, 33 with M protein alone, and 11 with polyclonal protein alone. There was no significant difference in incidence of the major clinical manifestations between the two groups with and without myeloma. They had a common characteristic histologic finding of the lymph node resembling that of Castlemans disease.

Nationwide survey of multiple sclerosis in Japan Clinical analysis of 1,084 cases

Between October 1972 and October 1973, the first nationwide survey of the multiple sclerosis group of diseases in Japan was performed by the Multiple Sclerosis Research Committee of Japan, supported by the Japan Ministry of Health and Welfare. Reports on 1,084 patients with the multiple sclerosis group were collected: 509 patients with multiple sclerosis, 82 with Devics disease, 357 with “multiple sclerosis possible,” and 136 with “other or unclassified demyelinating diseases.” The natural history in the present nationwide series of multiple sclerosis patients showed considerable similarity to that of patients in Western countries, suggesting that multiple sclerosis in Japan is essentially the same as that in the Western countries. However, the previously reported special characteristics of Japanese multiple sclerosis patients, namely, a higher rate of visual impairment at onset, a higher rate of optic nerve involvement during the course of illness, and a higher rate of Devics disease, were reconfirmed in the present series.

Videofluorographic Study of Swallowing in Parkinson's Disease

Abstract. We studied 16 patients with Parkinsons disease (PD) with dysphagia and 8 young and 7 elderly normal controls videofluorographically to evaluate the nature of swallowing disorders in PD patients. In 13 patients, abnormal findings in the oral phase were residue on the tongue or residue in the anterior and lateral sulci, repeated pumping tongue motion, uncontrolled bolus or premature loss of liquid, and piecemeal deglutition. Thirteen patients showed abnormal findings in the pharyngeal phase, including vallecular residue after swallow, residue in pyriform sinuses, and delayed onset of laryngeal elevation. Ten of these patients also showed abnormal findings in both the oral and pharyngeal phases. Aspiration was seen in 9 patients. The oral transit duration was significantly longer in the patients with and without aspiration than in the control subjects. The stage transition duration, pharyngeal transit duration, duration of the upper esophageal sphincter (UES) opening, and total swallow duration were significantly longer in the patients with and without aspiration than in the young controls, but were not longer than in the elderly controls. These durational changes in the pharyngeal phase of swallowing were similar to those in the elderly controls. The findings suggest that the disturbed motility in the oral phase of swallowing may be due to bradykinesia. Although PD patients with dysphagia evince a variety of swallowing abnormalities, the duration of pharyngeal swallowing may remain within the age-related range until the symptoms worsen.

Mitochondrial encenhdomyopathy with lachte‐pyruvate elevation and brain infarctions

We studied a patient with somatic growth failure with easy fatigability, myopathy with mitochondrial abnormality, increased lactate and pyruvate in blood and CSF, mental retardation, seizure, myoclonus, deafness, cerebellar ataxia, and blindness with macular degeneration and optic atrophy. Pathologic findings included multiple brain infarctions and massive calcification in the basal ganglia. Biochemical studies of isolated mitochondria revealed decreased oxygen consumption in skeletal muscle, diaphragm, and brain, suggesting an abnormality in the respiratory chain.

Immunological Studies of HTLV-I Associated Myelopathy

We report the immunological studies of 50 cases with a chronic progressive myelopathy associated with elevated antibodies to human T-cell lymphotropic virus type I (HTLV-I) and adult T-cell leukemia-like cells (HAM). These are as follows: (1) T-cell activation; (2) increase of the OKT4/OKT8 ratio, due mostly to increased inducer/helper T cells and/or decreased suppressor/cytotoxic T cells; (3) decrease of natural killer cell numbers and activity; and (4) increase of immunoglobulins (IgG, IgA). Our results provide evidence that autoimmune events may participate in the pathogenesis of HAM and may be relevant in the hypothesis of common immune mechanisms between HAM and HTLV-I positive tropical spastic paraparesis.

A case of nemaline myopathy with ophthalmoplegia and mitochondrial abnormalities

A case of nemaline myopathy with ophthalmoplegia is reported. The patient was a 35-year-old man born of consanguineous parents. He had a myopathic face, high-arched palate, nasal voice, scoliosis, very thin trunk and marked muscle weakness involving face, neck, limbs and trunk. He also had ptotis of the left eyelid and mild bilateral ophthalmoplegia, also detected by electrooculogram. Biopsy of gastrocnemius muscle revealed nemaline rods. At the ultrastructural level, the rods appeared to have axial and cross striations, and in cross-sections at high magnification they seemed to have a crystal lattice structure. Intranuclear rods were also observed. In addition to the rods, abnormal mitochondria including a number of paracrystalline inclusions were seen.

Pain-related somatosensory evoked potentials in dementia

Pain-related somatosensory evoked potentials (pain SEPs) were examined in 25 demented and non-demented patients to investigate the cognitive function for pain in the progression of dementia. Pain SEPs by CO2 laser stimulation were recorded together with auditory event-related potentials (auditory ERPs). P340 in pain SEPs and P300 in auditory ERPs were analysed. The latency of P300 evoked in mildly demented patients was inversely correlated with the Mini-Mental state examination score, and the latency of P340 was also inversely correlated to that score but to a lesser extent. Pain SEPs were not recorded in 4 of 7 severely demented patients. These results indicate that the P340 component of pain SEPs is apparently different from the P300 component of auditory ERPs and suggest that the pain perception in severely demented patients may be abnormal.

Neuro-ophthalmological abnormalities in HTLV-I associated myelopathy

To determine whether neuro-ophthalmological abnormality exists in HTLV-I Associated Myelopathy (HAM), 22 patients were examined using electrooculography and visual evoked potentials and compared with controls. Eleven cases had mild abnormalities in smooth pursuit movements. Of these 11 patients, four had transient diplopia or jerky eye movements. One patient had a delayed P100 latency in the visual evoked potentials. These data suggest that the central nervous system may also be involved in HAM.

Treatment of disseminated intravascular coagulation and its prodromal stage with gabaxate mesilate (FOY): a multi-center trial.

One hundred and ninety-one patients with disseminated intravascular coagulation syndrome (DIC) or its prodromal stage (preDIC) were treated with only gabaxate mesilate (FOY) (group G) or a combination of gabaxate and unfractionated heparin (group GH), and the efficacy of gabaxate was evaluated in a multicenter study. Following the treatment, the mean DIC score, which was evaluated on the basis of clinical symptoms and hemostatic parameters, decreased significantly to 5.58 +/- 3.48 from 6.75 +/- 3.14 in group G (p < 0.001) and to 6.34 +/- 3.33 from 7.31 +/- 3.00 in group GH (p < 0.05). In patients with overt DIC, the mean score decreased to 6.71 +/- 3.54 from 8.42 +/- 2.84 (p < 0.001). In DIC, the rate of overall efficacy was 46.2% in group G and 35.1% in group GH. In preDIC, it was 41.5% in group G and 27.3% in group GH. No side effects, including severe bleeding, were found in this study. The results indicate that gabaxate mesilate is clinically effective for patients with DIC and preDIC.

Immune dysfunction in hypophosphatemic vitamin D-resistant rickets: Immunoregulatory reaction of 1α(OH) vitamin D3

We investigated immunologic function in six cases with hypophosphatemic vitamin D-resistant rickets (VDRR) before and after treatment with 1 alpha-hydroxycholecalciferol (1 alpha(OH) vitamin D3). All cases suffered frequent episodes of infection, which tended to be more severe in the older patients. OKT9-, OKT10-, and OKM1-positive cells and adenosine deaminase (ADA) were significantly increased, whereas numbers and activity of natural killer (NK) cells were lower than normal before treatment. After administration of 1 alpha(OH) vitamin D3, however, the susceptibility to infection apparently decreased, and NK cell number and activity increased in all patients. ADA was also significantly decreased and remained in the normal range after treatment. These results suggest that vitamin D plays a role in the impaired immunoregulatory functions of NK cells in VDRR. Furthermore, ADA may be one parameter reflecting this immunologic impairment.